I-Ewing's sarcoma ibonakala ngokukhula kwesimila esiyingozi emathanjeni nasezicutshini ezithambile. Lesi simila sinesici sokuba namandla aphezulu e-metastatic. Kungakhathaliseki ukuthi ukusabalala kwamaseli e-tumor kuwo wonke umzimba kuvame ukuhlonzwa kule pathology.

Kuyisifo esiyivelakancane esihlasela kakhulu izingane. Isigameko sakhona sifinyelela ku-1/312 wezingane ezingaphansi kweminyaka engu-500 ubudala.

Iqembu lobudala elithinteke kakhulu ekukhuleni kwaleli fomu lesimila liphakathi kweminyaka engu-5 nengama-30 ubudala, kanti kube nokuqhamuka okukhulu kakhulu phakathi kweminyaka eyi-12 nengu-18 ubudala. (3)

Ukubonakaliswa okuhlobene nomtholampilo ubuhlungu nokuvuvukala endaweni yesimila.

Izindawo zamangqamuzana ezinwele zesici se-Ewing's sarcoma ziningi: imilenze, izingalo, izinyawo, izandla, isifuba, ukhalo, ugebhezi, umgogodla, njll.

Le Ewing sarcoma ibizwa nangokuthi: isimila sokuqala se-peripheral neuroectodermal. (1)

Ukuhlolwa kwezokwelapha kuvumela ukuxilongwa okungenzeka kwalesi sifo futhi kunqume isigaba saso sokuthuthuka. Ukuhlolwa okuhambisana kakhulu yi-biopsy.

Izici ezithile nezimo ezithile kungathinta ukubikezelwa kwalesi sifo endabeni ethintekile. (1)

Lezi zinto zifaka ikakhulukazi ukusabalala kwamangqamuzana ezimila emaphashini kuphela, isibikezelo saso esivumayo, noma ukwakheka kwamafomu e-metastatic kwezinye izingxenye zomzimba. Kulokhu kokugcina, isibikezelo sibi kakhulu.

Ngaphezu kwalokho, ubukhulu besimila kanye nobudala bomuntu othintekayo kunendima ebalulekile ekubikezelweni okubalulekile. Ngempela, esimweni lapho ubukhulu besimila bukhuphukela ngaphezu kuka-8 cm, isibikezelo siyakhathaza kakhulu. Ngokuphathelene neminyaka yobudala, ukuxilongwa kokuqala kwezifo kwenziwa, kungcono ukubikezelwa kwesiguli. (4)

I-Ewing's sarcoma ingenye yezinhlobo ezintathu eziyinhloko zomdlavuza wamathambo oyinhloko kanye ne-chondrosarcoma ne-osteosarcoma. (2)

Izimpawu ezivame ukuhlotshaniswa ne-Ewing's sarcoma ubuhlungu obubonakalayo nokuvuvukala emathanjeni athintekile nezicubu ezithambile.

 Ukubonakaliswa okulandelayo komtholampilo kungahle kuqhamuke ekukhuleni kwe-sarcoma enjalo: (1)

• ubuhlungu kanye / noma ukuvuvukala ezingalweni, emilenzeni, esifubeni, emuva noma okhalweni;
• ukuba khona “kwamaqhuqhuva” kulezi zingxenye ezifanayo zomzimba;
• ukutholakala komkhuhlane ngaphandle kwesizathu esithile;
• ukuphuka kwamathambo ngaphandle kwesizathu.

Izimpawu ezihambisanayo nokho zincike endaweni yesimila kanye nokubaluleka kwaso mayelana nokukhula.

Ubuhlungu obuzwa yisiguli ngalesi sifo buvama kakhulu ngokuhamba kwesikhathi.

 Ezinye, izimpawu ezingavamile kakhulu zingabonakala, njenge: (2)

• umkhuhlane omkhulu futhi ophikelelayo;
• ukuqina kwemisipha;
• ukuncipha okukhulu kwesisindo.

Kodwa-ke, isiguli esine-Ewing's sarcoma singase singabi nazo izimpawu. Ngalo mqondo, isimila singabe sesikhula ngaphandle kokubonakaliswa okuthile komtholampilo futhi ngaleyo ndlela sithinte ithambo noma izicubu ezithambile ngaphandle kokubonakala. Ingozi yokuphuka ibaluleke kakhulu esimweni sakamuva. (2)

Njengoba i-sarcoma ka-Ewing iyindlela yomdlavuza, kuncane okwaziwayo ngemvelaphi ngqo yokukhula kwayo.

I-hypothesis nokho yabekwa phambili maqondana nembangela yokuthuthuka kwayo. Ngempela, i-sarcoma ka-Ewing ithinta ikakhulukazi izingane ezineminyaka engaphezu kwengu-5 ubudala nentsha. Ngalo mqondo, kungenzeka ukuthi kube khona ukuxhumana phakathi kokukhula kwamathambo ngokushesha kulesi sigaba somuntu kanye nokuthuthukiswa kwe-sarcoma ka-Ewing.

Isikhathi sokuthomba ezinganeni nasebusheni senza amathambo nezicubu ezithambile zisengozini enkulu ekukhuleni kwesimila.

Ucwaningo luye lwabonisa nokuthi ingane ezalwe nge-hernia ye-umbilical inamathuba aphindwe kathathu okuthuthukisa i-sarcoma ka-Ewing. (2)

Ngale kwale mibono okukhulunywe ngayo ngenhla, kubekiwe nemvelaphi yobukhona bokudluliswa kofuzo. Lokhu kudluliselwa kuhilela isakhi sofuzo se-EWSRI (22q12.2). Ukudluliselwa kwe-A t (11; 22) (q24; q12) ngaphakathi kwalolu fuzo lwentshisekelo kutholwe cishe ku-90% wamathumba. Ukwengeza, izinhlobo eziningi zofuzo kube yizihloko zophenyo lwesayensi, okubandakanya izakhi zofuzo ze-ERG, ETV1, FLI1 kanye ne-NR4A3. (3)

Ngokombono lapho imvelaphi ngqo ye-pathology ikhona, kuze kube namuhla, namanje ayaziwa kahle, izici ezinobungozi nazo.

Ngaphezu kwalokho, ngokwemiphumela yocwaningo lwesayensi, ingane ezalwe ne-umbilical hernia ingase ibe namathuba aphindwe kathathu okuba nohlobo lomdlavuza.

Ngaphezu kwalokho, ezingeni lezakhi zofuzo, ukutholakala kokudluliswa ngaphakathi kohlobo lwe-EWSRI (22q12.2) noma okuhlukile kofuzo kuzakhi zofuzo ze-ERG, ETV1, FLI1 kanye ne-NR4A3, kungaba yisihloko sezinye izici ezinobungozi zokuthuthukisa lesi sifo. .

Ukuxilongwa kwe-sarcoma ka-Ewing kusekelwe ekutholeni umehluko ngokutholakala kwezimpawu zesiguli.

Ngemuva kokuhlaziywa kukadokotela ngezindawo ezibuhlungu nezivuvukele, kuvamise ukwenziwa i-x-ray. Ezinye izinhlelo zezithombe zezokwelapha zingasetshenziswa, njenge: I-Magnetic Reasoning Imaging (MRI) noma iskene.

I-biopsy yethambo nayo ingenziwa ukuqinisekisa noma cha ukuxilongwa. Kulokhu, isampula yomnkantsha wethambo ithathwa futhi ihlaziywe ngaphansi kwe-microscope. Lezi zindlela zokuxilonga zingenziwa ngemuva kwe-anesthesia evamile noma yendawo.

Ukutholakala kwalesi sifo kufanele kwenziwe ngokushesha okukhulu ukuze ukuphathwa kwenziwe ngokushesha futhi ngaleyo ndlela ukubikezela kungcono.

 Ukwelashwa kwe-sarcoma ka-Ewing kuyefana nokwelashwa okuvamile kweminye imidlavuza: (2)

• ukuhlinzwa kuyindlela ephumelelayo yokwelapha lolu hlobo lwe-sarcoma. Kodwa-ke, ukungenelela kokuhlinzwa kuya ngosayizi wesimila, indawo okuyo kanye nezinga lokusabalala kwayo. Umgomo wokuhlinzwa ukufaka ingxenye yethambo noma izicubu ezithambile ezilinyazwe isimila. Ngalokhu, i-prosthesis yensimbi noma i-bone graft ingasetshenziswa ukumiselela indawo ethintekile. Ezimweni ezimbi kakhulu, ukunqunywa izitho kwesinye isikhathi kuyadingeka ukuvimbela ukubuyela komdlavuza;
• chemotherapy, evame ukusetshenziswa ngemuva kokuhlinzwa ukunciphisa isimila nokwenza lula ukuphulukiswa.
• i-radiotherapy, ibuye isetshenziswe kaningi ngemuva kokulashwa ngamakhemikhali, ngaphambi noma ngemuva kokuhlinzwa ukunciphisa usayizi wesimila nokugwema ingozi yokubuyela emuva.